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[目的]探讨脑室外神经细胞瘤(extraventricular neurocytoma ,EVN)的病理形态学、免疫表型、鉴别诊断.[方法]对2例 EVN 的临床表现、影像学、组织形态、免疫组化等进行分析.[结果]2例 EVN 中男女各1例,年龄为21和32岁.发生在左额叶及鞍区各1例.组织学形态改变:均可见由具有少突胶质细胞样细胞(oligodendrocyte like cells ,OLC)构成的蜂窝状结构,局灶或弥漫的神经节细胞分化,毛细血管丰富,血管壁增厚伴透明样变性 . 2例肿瘤细胞弥漫表达 Syn ,NSE ,NeuN 和 S‐100,而 NF ,Olig‐2,GFAP 灶性阳性,Ki‐67阳性细胞<2%.[结论]EVN 与中枢神经细胞瘤相比,组织学形态更加多样,更容易被误诊其他含OLC 的肿瘤,特征性的组织学特点及免疫表型有助于鉴别.“,”Objective] To explore the pathomorphology ,immunophenotypes and differential diagnosis of extraventricular neurocytoma(EVN) .[Methods]The clinical manifestations ,image ,histomorphology and im‐munohistochemistry of 2 patients with EVN were analyzed .[Results] The 2 patients were 1 male and 1 fe‐male .The ages of patients were 21 and 32 years old ,respectively .The sites of involvement included left fron‐tal lobe in one patient and saddle area in one patient .Histomorphology showed that honeycomb structure com‐posed by oligodendrocyte like cells(OLC) ,focal or suffused ganglion cell differentiation ,rich capillary ,thick‐ened vessel wall with hyaloids degeneration were seen .Syn ,NSE ,NeuN and S‐100 were diffusively expressed in tumor cells of 2 patients .NF ,Olig‐2 and GFAP were locally positive .Ki‐67 positive cells were less than 2% .[Conclusion] Compared with the central neurocytoma ,EVN is more diverse in histomorphology ,and more easily misdiagnosed as other tumors with OLC .The characteristic histological features and immunohisto‐chemical findings may help for differential diagnosis .